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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
Disclosure Statement
STARVATION, DEATH, AND DESTRUCTION: THE BATTLEFIELD OF AVN
I have no industry relationships to disclose
I will not discuss off-label use of medications
Karyn Brundige, MSN CPNP
Learning Outcomes
Interesting Trivia
1. Identify three factors that contribute to the development of AVN in children with hematologic or oncologic disorders 2. Contrast evidence-based treatment strategies for childhood AVN based on disease staging
What is the most common cause of AVN of the femoral head during childhood?
Trauma Legg-Calvé-Perthes disease Slipped capital femoral epiphysis Steroid therapy Radiation therapy Sickle cell disease Gaucher disease Lupus (SLE)
Different Name, Same Game Pathophysiology
Avascular necrosis Osteonecrosis Aseptic osteonecrosis Atraumatic necrosis Ischemic bone necrosis
Bone is a living tissue that requires blood AVN = death of bone tissue due to lack of blood supply
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
Normal Bone Growth
Pathways to Necrosis
Break down: Resorption of old bone by osteoclasts Build: Formation of new bone by osteoblasts AVN = Breakdown faster than repair
Vascular occlusion
• Trauma, stress fracture • SCD, venous stasis
Intravascular coagulation
• Hypercoagulable state • Fat emboli
Primary cell death
Pathways to Necrosis
• Steroids • Radiation therapy
Hips, Shoulders, Knees and Toes
↓ Blood supply to bone (ischemia)
Infarction and necrosis of bone & marrow cells
Single terminal blood supply that lack collateral circulation, especially the epiphysis of the long bones Can also affect the elbows, wrists, ankles, clavicles, vertebrae and small bones in the hand and feet
Tiny breaks/collapse of bone components Destruction and collapse of joint Mechanical failure
Risk Factors
Sickle Cell Disease and AVN
Sickle cell disease
Steroids (ALL, HCST)
Orthopedic disorders
Trauma
Lupus
Infection
Coagulation disorders
Gaucher disease
Renal transplant
Radiation therapy
Malignancy
Biphosphonate use
Homozygous (SS) and heterozygous (SC) disease Acetabulum, head of femur, head of humerus Majority of affected hips are painful Symptomatic hips progress to collapse in 5 years
Joint replacement associated with
Higher rates of failure Higher rates of early replacement Higher rates of infection
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
ALL Risk Factors for AVN
Corticosteroids Age >10 years (girls 10-14 and boys >15)
ALL Risk Factors for AVN ?
Race (white>black; non-Hispanic>Hispanic) Female sex High BMI, especially at diagnosis Elevated lipid levels
Genetic mutations
Other chemotherapy agents
Steroid Therapy and AVN
Exact pathogenesis unknown
Age and AVN
Alteration in circulating lipids → microemboli in arteries Stimulate bone marrow fat cells to increase in size/number → blocks venous flow Effect vascular endothelial cells and smooth muscle cells → stasis → increased intraosseous pressure → necrosis
Maturing bones of adolescents, especially long bones that have late epiphyseal closure and contribute to pubertal growth, most susceptible
Often multi-joint involvement Most cases diagnosed within 1 year of starting treatment, but may occur many years later
HCST and AVN
AVN after allogeneic transplant (Li, 2014)
Nested case-control study; AVN (160)/control (478) Median time to develop AVN: 14 months Risk factors
Age ≥ 5 years at time of transplant Female gender Myeloablative conditioning regimen Chronic GVHD
Decreased risk
Epiphyseal closure results in elevated intraosseous pressure? Excessive metabolic activity in growth plates and bones during pubertal growth spurt make them more susceptible to hypoxic effects? Increased susceptibility caused by a markedly increased growth rate and hormonal changes?
HCST and AVN
AVN after allogeneic transplant (Sharma, 2012)
St. Jude Children’s Research Hospital prospective study post-transplant 30% of patients developed AVN of knees and/or hips 45% of lesions had at least 30% epiphyseal involvement Median time to develop AVN: 12.3 months Risk factors
>10 years old at time of transplant AVN on MRI prior to transplant
Knees most frequent site, but hips had greater severity
Non-malignant disease Reduced-intensity conditioning regimens
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
Clinical Symptoms Presentation
Pain is the most common presenting symptom
Physical Examination
Limp Antalgic gait Restricted ROM Tenderness around bone Joint deformity Muscle wasting
Often described as throbbing, deep, or intermittent May be referred to other areas Symptoms usually start with just weight bearing, then progress to at rest
Affected joints may be asymptomatic Inflammatory symptoms generally absent Sudden, severe onset of pain concerning for joint collapse
Degree of Disability
What part of the bone is affected How large an area is involved
>30% of articular surface of hips or knees
is associated with worse outcomes <30% of articular surface may heal without collapse
How effectively the bone rebuilds itself
What Modality is Best? Imaging and Staging
X-ray: Doesn’t identify early changes; may remain normal for months after symptoms first appear MRI: Most sensitive and specific method CT: Used to assess extent of disease and calcification; clearly shows articular deformity and bone collapse Bone scan
More sensitive than X-ray, less sensitive than MRI Increased uptake mainly due to increased activity of osteoblasts (bone formation)
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
Stage I
Stage II
X-ray: Not detectable MRI: Slight bone marrow edema or effusion; joint effusion most commonly appears in the knees Bone scan: Increased uptake mainly due to increased activity of osteoblasts (bone formation)
X-ray: Mixed osteopenia (bone mineral density lower than normal) or sclerosis MRI: Evidence of lesion, abnormality in bone tissue Bone scan: Increased uptake
Stage III
X-ray and MRI: Bone collapse of joint appears imminent
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
Stage IV
X-ray and MRI: Collapse of joint
Seek and You Will Find St. Jude’s Total XV Therapy (Kawedia, 2011) Symptomatic AVN usually diagnosed first year of therapy 17.6% symptomatic AVN at 6-12 months from diagnosis 53.6% asymptomatic AVN diagnosed at end of therapy 25% of patients with asymptomatic MRI changes early in treatment (by week 10) went on to develop symptoms, but Most symptomatic patients had mild-moderate symptoms, and only a minority required surgical treatment
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
Can AVN Reverse?
Management
Study of symptomatic AVN in children with ALL
Study of femoral head AVN in 48 children with ALL
Treatment
Goals
Stop bone damage Reduce pain Improve function
Considerations
Age Extent, stage and location of damage Health of patient Risk for recurrence of malignancy
40% had complete resolution of symptoms at 5 years 24 patients with radiology follow-up: 25% partially or completely reversible lesions, 54% stable lesions, 21% progressive lesions
>10 years (40 children): 19 progressed to joint collapse and required total hip replacement; 21 had no improvement at end of monitoring <10 years (8 children): none required hip replacement; 4 had significant improvement at end of monitoring
Non-Surgical Management
Inconsistent and limited success in preventing disease progression
Restrict weight-bearing exercise Physical therapy Medications: lipid-lowering drugs, anticoagulants, vasodilators, bisphosphonates, traditional Chinese medications Electromagnetic stimulation Hyperbaric oxygen
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
Surgical Management
Joint preserving procedures
Core decompression Bone grafts or bone marrow Osteotomy Arthrodesis
Joint replacement (arthroplasty)
Core Decompression
Core Decompression and ABMI
Autologous bone marrow implantation to induce bone repair in femoral head Procedure
60-120 mL bone marrow aspirated from anterior iliac crest Concentrated to 15 mL of bone marrow cells Multiple small core drillings in the femoral epiphysis Concentrated marrow slowly injected into the femoral head 24 hour admit for hydration and pain management No cast or brace; 2 month touch down weight-bearing on crutches
Osteotomy
Remove section of affected bone above or below a weight-bearing joint to change its shape in order to decrease the stress on it
Core Decompression and ABMI
Pediatric trial (Novais, 2015)
11 patients with SCD, 14 hips >90% decreased pain, >80% decreased progression to joint collapse
Adult trial (Daltro, 2015)
89 patients with SCD Historically 70% to 90% without intervention progress to collapse/osteoarthritis requiring arthroplasty within 5 years Only 3.4% did not achieve good results No collapse of femoral head in 60-month follow up
Arthroplasty
Pre-collapse of joint Single 8-10 mm core into necrotic lesion, or small diameter multiple 3 mm cores Pain relief Reduce intraosseous pressure Create open area for new blood vessels to grow Enhance new bone growth 70-80% success rate at 5 years (Novais, 2015)
Late stage when joint is destroyed (collapsed or severe arthritis) Damaged bone is removed and replaced with an artificial joint
Prolonged post procedure recovery includes extended restricted weight-bearing until healing occurs
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
Sohail
Steroids and COG Studies
What’s Up With Sohail?
Modifications for AVN
Do not modify
Maintenance
Hold for grade 2 or greater (symptomatic, limiting instrumental ADLs) Consider restarting if joint symptoms resolve and MRI shows normalization or significant improvement
Initial treatment
Induction or re-induction/delayed intensification
Steroids HELD Nighttime bracing and hip splinting (avoid abduction) Very active, no pain
3 months: Scans stable
6 months: Femoral head remodeling 10 months: Restarted steroids
Off therapy: Normal joint
Maddy
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
What’s Up With Maddy?
Symptoms were not responsive to physical therapy, restricted weightbearing or core decompression Total hip replacement at 14 years old Very active but now has multi-focal AVN in right knee, hip, and shoulders
Joshua
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APHON 42nd Annual Conference, 2018 Karyn Brundige MSN CPNP
Starvation, Death, and Destruction: The Battlefield of AVN
Ben
Take Home Messages
AVN results from loss of blood supply to the bone Most commonly affects ends of long bones (hips and knees) Risk factors in our population include sickle cell disease and high dose or prolonged steroids Older patients generally have progressive disease requiring surgical intervention
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